Mariette Xavier
Département de rhumatologie, Université Paris-Saclay, INSERM U1184, Centre d'immunologie des infections virales et des maladies auto-immunes, Assistance Publique-Hôpitaux de Paris (AP-HP), Hôpital Bicêtre, Service de Rhumatologie, 78 rue du Général Leclerc, 94275 Le Kremlin-Bicêtre, France.
Biol Aujourdhui. 2024;218(1-2):1-8. doi: 10.1051/jbio/2024003. Epub 2024 Jul 15.
Sjögren's disease (SjD) is a systemic autoimmune disorder characterized by a triad of key symptoms affecting almost all patients (salivary and lacrimal dryness, pain and fatigue) and extra-glandular systemic involvement affecting one to two-thirds of patients. Over the past decade, knowledge of the epidemiology, classification criteria, assessment of systemic activity and symptoms presented by patients has grown. In addition, advances in understanding the pathophysiology of SjD have enabled a more targeted therapeutic approach. Current management of SjD is based on EULAR treatment guidelines. But since these recommendations, new drugs targeting specific pathophysiological pathways of the disease, and essentially B lymphocyte activation, have shown efficacy in phase 2 trials. In this review, we will summarize the available evidence on systemic therapies, including: 1. advances in outcome assessment, 2. current evidence on targeted disease-modifying therapies and biologic drugs targeting primarily B lymphocytes, 3. an overview of promising drugs being tested in ongoing trials.
干燥综合征(SjD)是一种全身性自身免疫性疾病,其特征为几乎所有患者都会出现的三联征关键症状(唾液腺和泪腺干燥、疼痛和疲劳)以及三分之一至三分之二患者会出现的腺外系统性受累。在过去十年中,关于流行病学、分类标准、全身活动评估以及患者所表现症状的知识不断增加。此外,对干燥综合征病理生理学理解的进展使得治疗方法更具针对性。目前干燥综合征的管理基于欧洲抗风湿病联盟(EULAR)的治疗指南。但自这些建议发布以来,针对该疾病特定病理生理途径(主要是B淋巴细胞活化)的新药已在2期试验中显示出疗效。在本综述中,我们将总结关于全身治疗的现有证据,包括:1. 疗效评估的进展;2. 针对主要作用于B淋巴细胞的疾病改善性治疗和生物药物的现有证据;3. 正在进行的试验中正在测试的有前景药物的概述。